Immunomodulating agents such as anti–programmed cell death protein 1 (PD-1) have recently gained a crucial role in the treatment of numerous human malignant tumors.1 Cemiplimab is a novel PD-1 blocker approved for the treatment of locally advanced or metastatic cutaneous squamous cell carcinoma (cSCC) in patients who are not candidates for curative surgery or radiation.2 During the last few years, other anti–PD-1 agents such as pembrolizumab and nivolumab have occasionally been used off-label to manage cases of advanced cSCC.3 We describe 1 patient with recessive dystrophic epidermolysis bullosa (RDEB) and multiple cSCCs who was successfully treated with pembrolizumab.